Card Set Information
In what 2 diseases does heparan and dermatan sulfate accumulate?
What enzyme is deficient in Hurlers disease?
alpha L iduronidase
What enzyme is deficient in Hunter's syndrome?
Which enzyme is deficient in I cell disease?
are glycoproteins or proteoglycans branched?
Enzymes destined for lysosomes are tagged how?
Mannose 6 phosphate.
Mannose in ER
Phosphylation in Golgi Aparatus
O linked glycosidic bonds use what a.a?
serine, threonine, hydroxylysine
N linked glycosidic bonds use what a.a?
What is the major feature of complex class of oligosaccharides linked to a protein in glycoproteins?
O linked carbs are attached in glycoproteins are attached where?
N linked carbs are attached where?
What is needed?
In glycoproteins the following consensus sequence is attached to a protein: Asn-x-ser/Threonine
X can be any a.a except?
NANA(sialic acid) is incorporated into a glycoprotein using what high energy molecule
In O glycosidic linked carbs what sugar is used
In N linked glycosidic linked carbs, what sugar is used?
Which is more common, N or O linked?
Which one of the glucosaminoglycans is not sulfated and covalently linked to proteins?
What the function of Hyaluronic acid?
found in synovial fluid, vitreous humor, extracellular matrix
Which one of the GAGs does not have uronic acid?
Where is Dermatan sulfate found?
Chondroitin sulfates are found where?
What is the only intracellular GAG
What is the trisaccharide linkage in proteoglycans?
Why are the core proteins of proteoglycans rich in serine and threonine residues??
So that O linkages to carbs can occur
How are proteoglycan monomers held onto Hyaluronic acid?
What is the source of sulfate for proteoglycans?
L gulonate to ascorbate requires what acid that is absent in humans?
L gulono lactone oxidase
Sulfation and acetylation of proteoglycans occurs when?
after monosaccharides have been incorporated
What property of GAG explains their reaction?
they are negatively charged
What is the repeating disaccharide in a GAG?
Amino sugar-acidic sugar
Proteoglycans bind to what GAG?
Which gag does not have Uronic acid?
What takes place of uronic acid in Keratan sulfate?
Are GAGs hetero or homo polysaccharides?
What are the repeating disaccharides units in GAGs?
D-gluco or galactosamine and D- Gluco or galactoronic acid(Uronic acid)
Which is the GAG that has L sugars and and a variety of other sugars such as NANA?
What makes up the trihexoside linker protein?
In I cell disease what is not phosphorylated?
Mannose viA THE deficient enzyme GlcNac phosphotransferase
What is the relation between insulin and potassium?
Insulin sequesters potassium in cells
How is Hyperkalemia treated?
What leads to polyphagia in DM?
Lack of insulin prevents entry of glucose into satiety center
lack of entry of glucose disinhibits hunger center
hunger amongst plenty